Bernard Soulier or Hemophilia! - USMLE Forums
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  #1  
Old 04-28-2010
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Blood Bernard Soulier or Hemophilia!

guys look at this question:


A 10 year old boy is brought into the emergency department after falling from bicycle. he presents with a large, painfully swollen knee, aspiration shows gross hemarthrosis. on further questioning, the patients parents say he bruises easily and had an episode of prolonged bleeding after loosing a tooth 1 month ago. his maternal uncle had similar bleeding difficulties. after further testing, pt. is diagnosed with X-linked recessive disorder. which of the lab. schemes correspond to patient's disorder


choice platelet count bleedin time PT aPTT

A normal norm norm inc

B norm inc norm norm

C norm inc norm inc

D dec inc norm norm

E dec inc inc inc




i thought the answer to be A because i thought(still think) its Hemophilia A
but the correct answer given is B describing it as Bernard soullier or Glanzmann, please explain why isnt it hemophilia and why its bernard soullier or glanzman disease???
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Old 04-28-2010
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Unhappy It's not a good question

This is question is wrong. Glanzmann and Bernard Soulier are both autosomal recessive. The only thing that may point away from hemophilia is that the child should usually start bleeding at a bit younger age. But still the scenario is definitely of hemophilia (the maternal uncle, the X linked recessive, the hemarthrosis all pointing to it).

You triggered me to make this table
Bleeding Disorders Table
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Old 04-28-2010
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o thanks Lee... great table!!!

emm... why in late liver failure bleeding tym will be increased??? dat depends on platelets... didnt get dat point!! is dat because of von willebrand factor being less synthesised???
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Old 04-28-2010
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Info Platelet dysfunction

Quote:
Originally Posted by doctorF View Post
o thanks Lee... great table!!!

emm... why in late liver failure bleeding tym will be increased??? dat depends on platelets... didnt get dat point!! is dat because of von willebrand factor being less synthesised???
Patients with advanced liver disease have prolonged bleeding time even if their PT and PTT and platelet count is normal. The bleeding is due to platelet dysfunction rather than decrease in number
See this http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1663385/

Platelet dysfunction is also the reason behind prolonged bleeding time in uremia and patients on Aspirin.
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Old 07-13-2015
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I agree with friends,too. this is a typical Hemophillia case and the question is wrong.
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